'Young' men got Lou Gehrig's disease

For Navy Capt. Denis Army, the first clue came Sept. 21, 1998, when he stumbled while running the bases in a shipboard softball game. It was the day before the former college baseball player turned 45 and three days after his first anthrax shot.

Lt. Cmdr. Brody Prieto's problems began about a year after his only anthrax shot, at 31. His voice became too slurred to understand over a radio while flying missions as an F-14 radar-intercept officer in the Middle East.

Both Navy men from Virginia Beach were eventually diagnosed with amyotrophic lateral sclerosis - ALS, or Lou Gehrig's disease.

Both died thinking that their anthrax shots triggered - or caused - this always-fatal illness, despite military doctors' refusal to consider the possibility, their widows say.

Some scientists say there might be good reasons to think that the two men were right.

ALS involves degeneration of nerve cells in the brain and spinal column. Patients lose use of voluntary muscles, including those that help them stand, breathe and eat. It's a rare disease that typically kills within three to five years of diagnosis.

Army died in May 2000, after a particularly rapid form of ALS left him bedridden a few months after diagnosis.

Prieto was hit by a particularly fast form of ALS and given two years to live. He survived three and died in September.

In recent years, researchers have learned that military members are significantly more likely to get ALS than civilians. They also found that troops deployed in the 1999 Gulf War are more likely to get ALS - and get it at unusually early ages - than others in uniform.

CAUSE OF VET'S HIGH RATE OF ALS UNKNOWN

Nationwide, ALS usually hits men ages 60 to 80. Onset before 45 is rare, the definition of "young" in an important study of Gulf War vets. That makes Prieto's and Army's illnesses remarkable in themselves. A third member of the Navy died of the disease in 2001 at 29. Her symptoms began shortly after receiving anthrax vaccinations, her mother says.

Kelli Allen is co-investigator of the National Registry of Veterans with ALS, a government project that tracks victims and studies the issue. Allen says no one's sure why the disease hits young veterans this way. A small percentage of ALS cases are purely genetic. Researchers think that the others result from a combination of genetics and exposure to environmental factors, which might include chemicals or other substances.

The anthrax vaccine's first widespread use came during the Gulf War. Many veterans who complain that their health took a nose dive after the vaccine in more recent years have symptoms similar to those of Gulf War veterans. Some researchers think there's a link between the disease and vaccine.

Peter S. Spencer is co-author of one significant study linking Gulf War service with ALS. He's also director of the Center for Research on Occupational and Environmental Toxicology at Oregon Health and Science University in Portland. Spencer says the vaccine is one of many possibilities that should be considered in determining why veterans suffer ALS at high rates.

Further investigations are needed to narrow the list of possible causes, he says, and a paper outlining how that could be done was submitted to the Department of Veterans Affairs in 2002. So far, he says, he hasn't received a response.

"They need to see the light of day," Spencer says. "We were expecting to get a follow-up from the VA by now."

Allen says another study was financed first. "This is definitely not something that's been swept under the carpet," she says. The program "is trying to use an efficient strategy."

She acknowledges that this approach excludes people who have died, such as Army and Prieto, and might miss important data as a result.

Prieto - an All-American fencer and boxer before graduating from the Naval Academy in 1994 - logged a perfect score on physical fitness tests shortly before his voice problems, says his widow, Theresa Prieto.

Army was commander of the guided-missile cruiser Cape St. George when he stumbled, then learned that he couldn't stand on tiptoes. He was on track for admiral, exercised daily and was playing, coaching and refereeing competitive soccer and other sports until he fell ill, says his widow, Beth Army.

Neither man got ALS because of a known genetic inheritance.

NO SIMPLE TEST FOR MOST CASES OF ALS

ALS is diagnosed by eliminating other possible causes of the symptoms, not by a single yes-or-no test. Testing for genetic inheritance is easy, but saying anything definitive about environmental causes is impossible right now, Spencer says.

Many ALS researchers think that it takes at least five years from the time of a harmful exposure or event until symptoms are noticed. Robert Haley, who did the early-onset-ALS study among Gulf War veterans, says that's because it's thought it takes that long before enough motor neurons die and affect general muscle function.

A three-day or one-year progression from vaccination to disease symptoms might be too rapid, Spencer says, but "I have no basis to say it's impossible." That probably reflects more on what isn't known about ALS than anything, he says.

It's also possible that veterans like Army and Prieto have a similar disease or illness that doesn't fit the standard circumstances of ALS, he says.

Army was diagnosed with ALS despite spinal fluid and foot reflexes atypical of ALS, medical records show. In Prieto's case, loss of speech usually follows debilitating effects on arms and legs with ALS. The reverse is rare, but it happens.

Their unusual symptoms and rapid decline could indicate an unusual cause but might also be biologic chance, Spencer says.

Prieto and Army have something else in common, their widows say: Both refused to sign medical-discharge papers until Navy doctors included mention of anthrax vaccination. The two, who didn't know each other, thought that it might be important someday, the widows say.

Navy doctors continued to give anthrax vaccinations to Army even after his muscle problems began. All the shots were from vaccine lot FAV030 - a 200,000-dose batch of the drug that the Food and Drug Administration later found was contaminated with an oil called squalene, medical records show.

Doctors at civilian clinics specializing in treating troops with illnesses typical of autoimmune diseases say they've linked the symptoms to people who got doses from FAV030 and other doses of anthrax vaccine with squalene. Autoimmune disease, in which the body's immune system attacks and destroys healthy cells and tissue, is one of several theories on how ALS destroys nerve cells.

Squalene has been shown to cause severe autoimmune problems in laboratory animals, and an Army experiment in the 1940s with vaccine containing squalene resulted in several similar illnesses.

Pam Asa, a researcher at Tulane University in New Orleans, says the exact number of vets who became ill after receiving vaccines with squalene isn't known. So far, the government hasn't been willing to finance research to find out.

A government study last year found that troops inoculated with vaccine from FAV030 had a higher rate of disability than those inoculated from other lots. But Col. John Grabenstein, a co-author of the study and director of the anthrax vaccine program, says there's insufficient proof of problems with FAV030.

SOURCE OF SQUALENE IN VACCINE UNKNOWN

The military and manufacturer say they don't know how squalene got there. The military's Web site dismisses the problem by noting that squalene is an essential, naturally occurring oil in humans and that the quantities detected were too small to matter. FDA officials agree but told Congress that they didn't have data to be sure.

Asa and others point out that the military has been experimenting with squalene to increase the potency of vaccines for more than 50 years - including anthrax vaccine before the Gulf War.

Grabenstein says data from 1998 through 2000 - the last time that post-vaccination records were analyzed for anthrax - indicates no reason to think that anthrax vaccinations lead to more cases of ALS. That study might have looked at too short a period to detect ALS cases, however, because it covered very few people who'd been inoculated for more than a year.

A report filed earlier this year with the FDA asks for an investigation into the relationship between the vaccine and ALS. It cites three cases that weren't reported by the military or the drug's manufacturer to the Vaccine Adverse Events Reporting System, or VAERS.

The third case involves Petty Officer 2nd Class Kristin Shemeley, who died at 29 - less than three years after three doses of anthrax vaccine with squalene. She lost use of the arm she'd been vaccinated in a few months after starting the shots, then became progressively worse, says a sworn legal document about her illness.

High-ranking admirals, generals and a member of Congress visited Shemeley at Walter Reed Army Medical Center in Washington, says her mother, Ginger Shemeley of Quakertown, Pa. Kristin told many of them her illness resulted from the vaccine, her mother says.

No one from the military reported Kristin's illness to VAERS. Ginger Shemeley did.

VAERS compiles reports about illnesses and injuries after administration of any licensed vaccine, to help identify problems. A VAERS report isn't proof of cause and effect. That's determined only after a number of similar cases are noticed in the database, followed by research.

To encourage more reports, a patient, relative or doctor can file with VAERS - but few outside the medical field know about it.

Beth Army filed the VAERS report on her husband in August.

Theresa Prieto, a widow with three young children, says she plans to file a report soon.